Resumen

GARCIA, Blanca; FERRER, María Solange  y  ACOSTA, Geylor. Tumor maligno de la vaina de nervio periférico y neurofibromatosis. Rev. venez. oncol. [online]. 2008, vol.20, n.1, pp.38-41. ISSN 0798-0582.

We report a case 29 year old female with history of neurofibromatosis and local resection of a left flank tumor. The pathology of surgical specimen reports malignant mesenchymal neoplasm. Two months after the procedure refers rapidly growing tumor located underneath the surgical scar. Physical examination: tumor in left lower quadrant of the abdomen, firm, with ulcerated skin. The magnetic nuclear resonance reveals retroperitoneal tumor extending into abdominal cavity, displacing aorta, vena cava, left kidney, left urether, descending colon. Laparotomy is performed, a retroperitoneal tumor infiltrating abdominal wall and skin, lobulated. The pathology and immunohistochemistry stains with S-100 protein and LEU 7 was compatible with peripheral malignant nerve sheath tumor associated neurofibromatosis malignant high grade neoplasia. Five year survival rates are low: 15 % - 30 %. Management of these tumors includes wide local resection with intraoperative frozen section of surgical margins, postoperative external beam radiotherapy and chemotherapy for metastatic disease.

Palabras clave : Tumor; nerve sheath; neurofibromatosis; mesenchymal.